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Bowel perforation from malignant atrophic papulosis treated with eculizumab

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Abstract We present the case of a 57-year-old man with known cutaneous manifestations of malignant atrophic papulosis, also known as Köhlmeier-Degos disease, who developed an almost-fatal small bowel perforation following… Click to show full abstract

Abstract We present the case of a 57-year-old man with known cutaneous manifestations of malignant atrophic papulosis, also known as Köhlmeier-Degos disease, who developed an almost-fatal small bowel perforation following a parathyroidectomy. He required two surgical interventions during his initial hospitalization and was started on eculizumab. Despite these therapies, the patient developed recurrent bowel perforations and ultimately died.

Keywords: bowel perforation; atrophic papulosis; malignant atrophic

Journal Title: Baylor University Medical Center Proceedings
Year Published: 2020

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